TY - JOUR
T1 - Liver transplantation for Wilson's disease: The burden of neurological and psychiatric disorders
AU - Medici, V
AU - Mirante, Vg
AU - Fassati, Lr
AU - Pompili, Maurizio
AU - Forti, D
AU - Del Gaudio, M
AU - Trevisan, Cp
AU - Cillo, U
AU - Sturniolo, Gc
AU - Fagiuoli, S.
PY - 2005
Y1 - 2005
N2 - A retrospective data analysis on liver transplantation for Wilson's disease (WD) was performed among Italian Liver Transplant Centers. Thirty-seven cases were identified. The main indication for liver transplantation was chronic advanced liver disease in 78% of patients. Mixed hepatic and neuropsychiatric symptoms were recorded in 32.3%. Eight patients presented with fulminant liver failure; 44.8% were on medical treatment. Patient and graft survival at 3 months, 12 months, 3 years, 5 years, and 10 years after transplantation were, respectively, 91.8%, 89.1%, 82.9%, 75.6%, and 58.8%, and 85.3%, 83.0%, 77.1%, 70.3%, and 47.2%. Neurological symptoms significantly improved after orthotopic liver transplantation (OLT), but the survival of patients with mixed hepatic and neuropsychiatric involvement was significantly lower than in patients with liver disease alone (P = 0.04). WD characterized by hepatic involvement alone is a rare but good indication for liver transplantation when specific medical therapy fails. Patients with neuropsychiatric signs have a significantly shorter survival even though liver transplantation has a positive impact on neurological symptoms. In conclusion, a combination of hepatic and neuropsychiatric conditions deserves careful neurological evaluation, which should contraindicate OLT in case of severe neurological impairment.
AB - A retrospective data analysis on liver transplantation for Wilson's disease (WD) was performed among Italian Liver Transplant Centers. Thirty-seven cases were identified. The main indication for liver transplantation was chronic advanced liver disease in 78% of patients. Mixed hepatic and neuropsychiatric symptoms were recorded in 32.3%. Eight patients presented with fulminant liver failure; 44.8% were on medical treatment. Patient and graft survival at 3 months, 12 months, 3 years, 5 years, and 10 years after transplantation were, respectively, 91.8%, 89.1%, 82.9%, 75.6%, and 58.8%, and 85.3%, 83.0%, 77.1%, 70.3%, and 47.2%. Neurological symptoms significantly improved after orthotopic liver transplantation (OLT), but the survival of patients with mixed hepatic and neuropsychiatric involvement was significantly lower than in patients with liver disease alone (P = 0.04). WD characterized by hepatic involvement alone is a rare but good indication for liver transplantation when specific medical therapy fails. Patients with neuropsychiatric signs have a significantly shorter survival even though liver transplantation has a positive impact on neurological symptoms. In conclusion, a combination of hepatic and neuropsychiatric conditions deserves careful neurological evaluation, which should contraindicate OLT in case of severe neurological impairment.
KW - Adult
KW - Female
KW - Hepatolenticular Degeneration
KW - Humans
KW - Liver Transplantation
KW - Male
KW - Mental Disorders
KW - Nervous System Diseases
KW - Retrospective Studies
KW - Survival Analysis
KW - Treatment Outcome
KW - Adult
KW - Female
KW - Hepatolenticular Degeneration
KW - Humans
KW - Liver Transplantation
KW - Male
KW - Mental Disorders
KW - Nervous System Diseases
KW - Retrospective Studies
KW - Survival Analysis
KW - Treatment Outcome
UR - https://publicatt.unicatt.it/handle/10807/14251
UR - https://www.scopus.com/inward/citedby.uri?partnerID=HzOxMe3b&scp=25144471713&origin=inward
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=25144471713&origin=inward
U2 - 10.1002/lt.20486
DO - 10.1002/lt.20486
M3 - Article
SN - 1527-6465
VL - 11
SP - 1056
EP - 1063
JO - Liver Transplantation
JF - Liver Transplantation
IS - 9
ER -